Mid-Systolic Notching of the Pulmonary Valve Doppler Signal is Highly Associated with Pulmonary Hypertension.

Mid-systolic notching (MSN) of the pulmonary valve Doppler signal represents a reflected systolic pressure wave from the pulmonary vasculature and is often seen in pulmonary hypertension (PH). We hypothesize that MSN is associated with a higher pulmonary vascular resistance (PVR) and mean pulmonary artery pressure (mPAP), and a diagnosis of PH in pediatric patients. This was a retrospective study of patients ≤ 18 years who had an echocardiogram obtained ≤ 30 days before catheterization for suspected PH. MSN was defined as an indentation in the initial two thirds of the systolic Doppler signal. PH was defined as mPAP > 20 mmHg and PVR ≥ 3.0 Wu  m2. Subgroups (MSN vs. normal) were compared. Receiver operator characteristic determined a continuous variable's discriminatory ability for a diagnosis of PH. Reproducibility of MSN was assessed. In total, 90 patients (73 with congenital heart disease) were included, of which 36 had MSN and 54 were normal. MSN patients were more likely to have PH, and had significantly higher mPAP, PVR, and lower pulmonary stroke volume. The presence of MSN had good discriminatory ability for PH diagnosis. The presence of MSN had high specificity (96%) for PH, whereas sensitivity was lower (54%). Reproducibility was 100% for MSN. MSN is a simple, highly reproducible echocardiographic metric associated with higher mPAP and PVR. When present, there is a high likelihood a diagnosis of PH confirmed by catheterization. Incorporation of MSN into imaging protocols may be useful. MSN appears worthy of further investigation in pediatric patients with suspected PH.

Left heart hypoplasia in the fetus: Echocardiographic predictors of outcome.

INTRODUCTION: Fetal left heart hypoplasia (LHH) with an apex-forming left ventricle may require neonatal intervention but it is difficult to predict. METHODS: We performed a retrospective study of fetuses with LHH defined as normal segmental anatomy, apex-/near-apex forming left ventricle, and ≥1 left-sided z-score ≤ -2 between 1997 and 2014. Fetuses with mitral or aortic atresia, critical aortic stenosis, extracardiac anomalies, or fetal intervention were excluded. Classification and regression tree analyses (CART) were performed to construct algorithms to predict postnatal circulation: no surgery versus biventricular surgery versus single ventricle (SV) palliation. RESULTS: Among 138 included fetuses, 52 (37%) underwent neonatal surgery, with 10 (7%) undergoing SV palliation. The strongest single outcome discriminator was exclusively left-to-right flow foramen ovale (FO) flow ≥32 weeks gestational age (GA) (seen in 0% with no surgery, 22% with biventricular surgery, 88% with SV palliation). On CART analysis >32 weeks GA, fetuses with right-to-left FO flow and aortopulmonary ratio >0.76 had 0% probability of neonatal surgery, while those with left-to-right FO flow and mitral valve z-score < -3.6 had a 70% probability of SV palliation. CONCLUSION: SV palliation is an uncommon outcome of fetal LHH. Fetal FO flow and other echocardiographic measures can help determine risk and type of postnatal intervention.

Single systemic right ventricle longitudinal strain: Intravendor reproducibility and intervendor agreement in children.

PURPOSE: Strain derived from speckle-tracking echocardiography is emerging as a useful tool in the assessment of single ventricle function. The purpose of this study is to compare layer-specific longitudinal strain values in children with single, systemic right ventricles (sRV) using two commercially available software platforms (GE EchoPAC (EP) and TomTec (TT)). METHODS: Two readers analyzed two-dimensional longitudinal strain on EP (v 202) and TT (v 2.21.25) in 40 pediatric sRV patients. Intravendor reproducibility and intervendor agreement between layer-specific measurements were assessed by intraclass correlation coefficient and Bland-Altman analysis. Absolute difference (AbΔ) and relative mean errors (RME) were calculated. Subgroup comparisons (stratified by age, heart rate (HR), and frames per second (FPS): HR ratio) were made. RESULTS: Median age was 4.4 years. 32 (80%) patients had hypoplastic left heart syndrome; 19 (48%) were post-Fontan. Intravendor reproducibility was excellent with high ICC (0.86-0.97). AbΔ between readers was small (1.2%-1.5%) with interobserver RME slightly higher for TT (11%-12% vs 8%-9% for EP). Layer-specific intervendor agreement was poor (ICC 0.45-0.62). Default layer comparisons (EP mid vs TT endo) showed good agreement (ICC 0.72-0.77) and less variability (AbΔ 2%, RME 15%) than layer-to-layer. There were no differences in ICC for groups dichotomized by age, HR, or FPS:HR ratio. sRV strain values are more negative when using EP. CONCLUSION: Intravendor reproducibility for sRV peak longitudinal strain in children is excellent with acceptable variability between experienced users. Intervendor, layer-specific strain agreement is poor. Vendor default layer strain values show better agreement but are not interchangeable.

Assessing the atria in pediatric sickle cell disease: Beyond the dilation.

BACKGROUND: Diastolic dysfunction (DD) and pulmonary hypertension (PH) are common causes of mortality for sickle cell disease (SCD) patients in developed countries. We hypothesized that left and right atrial strain (LAS-Ɛ, RAS-rƐ) are decreased in SCD adolescents, and that worsening values correlate with laboratory markers of disease severity. METHODS: Prospective cohort study of patients with HbSS genotype of SCD was compared with healthy controls. LAS and RAS were measured from 4- and 2-chamber views by a blinded reader. Peak strain and strain rate values were obtained for atrial contraction (ac), reservoir (res), and conduit (con) phases. Mitral/tricuspid Doppler velocities, left atrial volume, right atrial area were obtained. Laboratory variables were obtained from the electronic record with the three prior values being averaged. Differences in variables were assessed with Wilcoxon rank sum test, and correlations assessed with Spearman's coefficient. RESULTS: There were 33 SCD patients compared to 35 healthy controls of similar age, gender, and size. SCD patients had increased left atrial volume and right atrial area. For LAS, Ɛres was significantly lower in SCD patients. For RAS, RƐcon was significantly lower. Neither measurement correlated with clinical markers. The majority of SCD patients had relatively normal atrial strain values. Those with markedly lower values had similar atrial size. CONCLUSIONS: A sub-set of SCD patients have markedly low Ɛres and rƐcon. No correlation with clinical markers was identified. Larger, longitudinal studies may determine utility of atrial strain as a screening tool in this at-risk population.

Fatal cardiac arrest in pediatric heart transplant recipients: Query of the UNOS database.

The incidence of death by CA after PHTx is unknown. We aimed to determine the incidence and factors for fatal CA after PHTx, and whether a PM affects survival. Retrospective cohort study utilizing the United Network of Organ Sharing registry of patients transplanted ≤18 years. Multivariable analyses in hazard-function domain and Kaplan-Meier analyses were performed for an outcome of death due to CA. There were 7719 PHTx patients queried. CA was the reported cause of death in 11%. Age ≥13 years at time of transplant, presence of a PM, and depressed EF were identified as significant factors for fatal CA. Death due to CA beyond 10 years post-transplant was associated with depressed EF, CAV, and presence of a PM. Kaplan-Meier analysis demonstrated higher likelihood of fatal CA in patients with CAV and in those with a PM vs those without. In total, 15% of patients with a PM died from CA. CA is a relatively common cause of death after PHTx. The benefit of a PM remains unclear, but its presence does not confer complete protection. Patients with associated factors warrant vigilant surveillance and consideration for retransplantation.

The impact of fetal endoscopic tracheal occlusion in isolated left-sided congenital diaphragmatic hernia on left-sided cardiac dimensions.

OBJECTIVES: Fetal endoscopic tracheal occlusion (FETO) is offered to fetuses with congenital diaphragmatic hernia (CDH) and severe lung hypoplasia to promote lung growth and may secondarily affect left heart growth. The effects of FETO on left heart hypoplasia (LHH) are not described post-CDH repair. METHODS: A retrospective analysis was performed for fetuses with left-sided CDH who underwent FETO and severity-matched controls from 2007 to 2016 at our institution. Echocardiographic, ultrasound, and MRI data were reviewed. Left heart dimensions were assessed prenatally and postnatally. Primary clinical outcome evaluated was death. RESULTS: Twelve FETO patients and 18 controls were identified. Fetal LHH was noted in both groups and worsened after FETO. Postnatal mitral valve dimensions were larger in the FETO group pre-CDH repair (P = .03). Post-CDH repair, mitral valve and left ventricular dimensions were not significantly different between groups (P = .79 and P = .63 respectively) while FETO aortic valve dimensions were smaller (P = .04). Extracorporeal membrane oxygenation use was lower in the FETO group. No associations were found between left heart dimensions and outcomes. CONCLUSION: Although increased lung growth was seen after FETO, fetal LHH persisted with relative normalization seen post-repair. Persistent LHH post-FETO could be secondary to a small contribution of pulmonary venous return to the fetal left heart and increased intrathoracic pressures post-FETO.

Percutaneous Pulmonary Valve Implantation-Associated Ventricular Tachycardia in Congenital Heart Disease.

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is utilized for dysfunctional right ventricular outflow tracts (RVOT) to relieve obstruction, regurgitation, and RV dysfunction. PPVI has not been reported to induce arrhythmias. This study is the first to report the incidence of ventricular tachycardia (VT) after PPVI. METHODS: This was a retrospective study of all patients who had PPVI at a single institution. All patients were admitted after PPVI for overnight telemetry monitoring. Patients with no prior history of VT and newly detected VT within 24 hours post-PPVI were considered to have VT related to PPVI. RESULTS: In total, 79 patients had PPVI (age 17 ± 9 years, 66% tetralogy of Fallot/pulmonary atresia). PPVI-related VT was detected in 6 patients (7.5%). These patients had a lower BMI (17.5 ± 2.0 vs. 23.1 ± 6.6, P = 0.04). There was no difference in age, native conduit or percutaneous valve size, or change in the minimum diameter of the RVOT from pre- to post-PPVI. In the 6 patients, VT was non-sustained and monomorphic at rates between 120 and 170. Five started on ß-blockers. No patient required surgical explantation of the valve. Over a median follow-up of 2 years, 4 have weaned off medications and all are free of recurrence of VT with normal Holters. CONCLUSIONS: PPVI may be associated with transient VT in the acute peri-procedural period. Patients of smaller size may be more susceptible. All patients were managed conservatively and none of the patients had a recurrence, which is suggestive of a transient phenomenon.

Sudden cardiac arrest during sex in patients with either catecholaminergic polymorphic ventricular tachycardia or long-QT syndrome: a rare but shocking experience.

BACKGROUND: Patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) and long-QT syndrome (LQTS) are susceptible to cardiac events during sympathetic nervous system activation. Herein, we sought to determine the risk of cardiac events associated with sex in CPVT and LQTS patients. METHODS AND RESULTS: We reviewed the electronic medical record of patients seen in the Genetic Heart Rhythm Clinic. There were 445 patients ≥ 18 years diagnosed with LQTS (N = 402, age at diagnosis 30 ± 16 years) or CPVT (N = 43, age at diagnosis 25 ± 15 years). No sex-induced cardiac events occurred in the LQTS population, and 2 occurred in the CPVT population. Sex-induced events were more likely in CPVT (2/43, 4.7%) than LQTS (0/402, 0%, P = 0.008). One case involved a 22-year-old CPVT1 female with prior cardiac arrest, who experienced several appropriate implantable cardioverter defibrillator shocks during intercourse while taking ß-blockers. The second case was a 52-year-old CPVT1 male with history of recurrent exercise-triggered syncope, who had syncope during sex in the setting of ß-blocker noncompliance. Extrapolating from published estimates of intercourse frequency by age, the overall event rate was only 0.0004%, and 0.005% among the CPVT cohort. CONCLUSIONS: Potentially life-threatening cardiac events during sex in patients with CPVT are rare and even rarer in LQTS. Overall, the cardiac event per intercourse rate is extremely low. Patients and their partners should be reassured that sex is a low-risk activity from a cardiac standpoint.

Septum primum atrial septal defect in an infant with hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is a form of congenital heart disease characterized by severe underdevelopment of the left heart, leading to inadequate systemic blood flow. Several different atrial septal morphologies are observed in HLHS, most commonly a secundum atrial septal defect, patent foramen ovale, intact septum, and leftward displacement of the superior attachment of the septum primum. It has been postulated that atrial septal development is associated with the development of the left heart. We present a case of a newborn infant with HLHS and the unusual finding of a primum ASD.

Effect of percutaneous atrial septal defect and patent foramen ovale device closure on degree of aortic regurgitation.

BACKGROUND: In recent years, percutaneous device closure has become commonplace for treatment of atrial septal defect (ASD) and patent foramen ovale (PFO). However, the effect of device closure on aortic valve regurgitation (AR) has been questioned. OBJECTIVE: The purpose of this study was to define changes in AR using echocardiography, before and after device closure of ASD or PFO. METHODS: We retrospectively reviewed all patients who had percutaneous device closure of ASD or PFO at our institution between 2005 and 2009. We compared the preprocedure echocardiogram to the most recent follow-up study. All investigators were blinded to referral diagnosis, patient demographics, and size and type of the device placed. Aortic regurgitation was graded as none/trivial, mild, moderate, or severe. RESULTS: Overall, 322 patients qualified for inclusion; 204 with PFO and 118 with ASD. Mean age at time of closure was 48 ± 20 years (range 3-85 years). Mean duration of follow-up was 1.2 years (range 2 months to 5 years). Only two patients (0.6%) demonstrated any appreciable increase in AR severity. However, both of these patients manifested a clinically insignificant change from a trivial to mild grade of aortic regurgitation. CONCLUSIONS: Percutaneous device closure of PFO or ASD does not increase the incidence or severity of AR over medium-term follow-up.

Posterior reversible encephalopathy syndrome and hemorrhage associated with tacrolimus in a pediatric heart transplantation recipient.

Posterior reversible encephalopathy syndrome (PRES) is a disorder characterized by gray and white matter abnormalities in the temporal, parietal, and occipital lobes of the brain. Its etiology has been attributed to renal failure, immunosuppressive drugs such as cyclosporine and tacrolimus, and other potential entities leading to acute hypertension. Clinical findings include headaches, altered mental status, seizures, visual changes, and focal neurologic deficits. We report the case of a child who developed PRES with intracerebral and subarachnoid hemorrhages associated with tacrolimus exposure 10 days after heart transplantation for restrictive cardiomyopathy. The patient initially presented with complex partial seizures, headache, agitation, and hypertension. Head MRI was suggestive of PRES along with intracerebral and subarachnoid hemorrhages. Tacrolimus was discontinued and blood pressure was controlled. The patient's encephalopathy resolved, but he has had ongoing neurologic symptoms secondary to hemorrhage. Generally, PRES is less common in children than in the adult population and is a rare complication of calcineurin inhibitors (CNI). Presentation with secondary hemorrhage also can occur. In children receiving CNIs presenting with new neurologic symptoms, PRES should be considered as prompt discontinuation of the offending agent can induce resolution of symptoms. Children can develop hemorrhage in the context of PRES, leading to increased morbidity.

Empiric switch from calcineurin inhibitor to sirolimus-based immunosuppression in pediatric heart transplantation recipients.

Sirolimus is used in heart transplant patients with CAV and CNI-induced nephropathy. However, little is known regarding the tolerability, rejection rate, and effect on renal function when used empirically in children. We describe our experience with the empiric use of a sirolimus-based immunosuppressive regimen in pediatric heart transplantation recipients. We reviewed records of patients in whom conversion was attempted to a CNI-free sirolimus-based regimen. Rejection episodes and measures of renal function were recorded. We attempted to convert 20 patients, of which 16 were successful. In total, six of 20 patients (30%) experienced adverse effects. Of the 16 converted, four patients converted to sirolimus due to CNI-induced disease (three nephropathy, one CAV), while 12 patients (mean age 5.5 yr, range 0.1-21 yr; 33% female; 33% with a history of congenital heart disease) were empirically switched to sirolimus at a mean of 2.3 yr after transplant. Follow-up was available for a mean of 2.5 yr after conversion (range 0.5-8.3 yr). The rate of rejection while taking CNIs was 0.18 rejection episodes per patient-year (total of five episodes), compared with 0.03 rejection episodes per patient-year (total of one episode) while on sirolimus. Renal function, in terms of GFR, significantly improved after sirolimus conversion at latest follow-up (from 86 ± 37 mL/min to 130 ± 49 mL/min, p = 0.02). Here, we demonstrate the potential benefit of empiric use of sirolimus in pediatric heart transplant patients in a CNI-free regimen. Larger and longer studies are needed to further clarify risks of rejection and adverse effect profiles.

Correlating decline in left ventricular ejection fraction and longitudinal strain in pediatric cancer patients.

PURPOSE: Left ventricular ejection fraction (LVEF) is routinely used to monitor cardiac function in cancer patients. Global longitudinal strain (GLS) detects subclinical myocardial dysfunction. There is no consensus on what constitutes a significant change in GLS in pediatric cancer patients. We aim to determine the change in GLS associated with a simultaneous decline in LVEF in pediatric cancer patients. METHODS: This is a retrospective longitudinal study of pediatric cancer patients treated with anthracyclines between October 2017 and November 2019. GLS was measured by 2-dimensional speckle tracking. The study outcome was a decline in LVEF, defined as a decrease in LVEF of ≥ 10% points from baseline or LVEF < 55%. We evaluated two echocardiograms per patient, one baseline, and one follow-up. The follow-up echocardiogram was either (1) the first study that met the outcome or (2) the last echocardiogram available in patients without the outcome. Statistical analyses included receiver operator characteristic curves and univariable and multivariable Cox proportional hazards regression. RESULTS: Out of 161 patients, 33 (20.5%) had a decline in LVEF within one year of follow-up. GLS reduction by ≥ 15% from baseline and follow-up GLS >-18% had sensitivities of 85% and 78%, respectively, and specificities of 86% and 83%, respectively, to detect LVEF decline. GLS reduction by ≥ 15% from baseline and follow-up GLS >-18% were independently associated with simultaneous LVEF decline [hazard ratio (95% confidence intervals): 16.71 (5.47-51.06), and 12.83 (4.62-35.63), respectively]. CONCLUSION: Monitoring GLS validates the decline in LVEF in pediatric cancer patients.

Relation of Right Atrial Strain to Mortality in Infants With Single Right Ventricles.

We explored associations of surveillance testing in infants with single right ventricle (sRV) physiology with clinical outcomes. This prospective, single-center study included patients with sRV who had initial palliative surgery (September 2019 to December 2020). Echocardiograms and B-type naturetic peptide (BNP) obtained as a pair within 24 hours as part of clinical care were included. The primary outcome was death/heart transplant. Secondary outcomes included interstage duration of milrinone use, hospital length of stay, and no digoxin use. sRV functional assessment (subjective grade, fractional area change, tricuspid annular plane systolic excursion, global longitudinal strain, right atrial strain [RAS]) was performed offline. Associations between echocardiography, BNP, and clinical outcomes were determined. Of 26 subjects (47 encounters), 20 had hypoplastic left heart syndrome (77%). Median age at data collection was 50 days (interquartile range 26 to 90). In most encounters (73%), sRV function was subjectively normal. Median BNP was 332 pg/ml (interquartile range 160 to 1,085). A total of 5 patients (19%) met the primary outcome and had lower RAS (14.1 vs 21.3, p = 0.038), but all other parameters were similar to transplant-free survivors. RAS (16.1%, 0.83) had the highest area under curve, followed by global longitudinal strain (-14.4%, 0.77). Higher RAS was associated with fewer days on milrinone (coefficient -1.37, 95% confidence interval [CI] -2.54 to -0.20, p = 0.02) and higher odds of digoxin use (odds ratio 1.09, 95% CI 1.01 to 1.18, p = 0.047). Higher BNP was only associated with a lower odds of digoxin use (odds ratio 0.69, 95% CI 0.5 to 0.96, p = 0.03). In conclusion, RAS is a potentially important imaging marker in infants with sRV and merits further investigation in larger studies.

Does Cardiac Catheterization Facilitate Hemodynamic Optimization of Pediatric Patients on Continuous-Flow Ventricular Assist Devices?

Understanding optimal ventricular assist device (VAD) parameters for pediatric patients is valuable given the inherent issue of patient-device size mismatch and heterogeneous cardiac anatomy in children. We evaluated our center's experience of continuous-flow VAD (CF-VAD) optimization using cardiac catheterization. We performed a retrospective analysis of all patients on CF-VAD support who underwent hemodynamic heart catheterization from 2013 to 2018. Fifteen patients had 16 hemodynamic catheterizations performed. The indications for hemodynamic optimization by catheterization included clinical signs of heart failure while on CF-VAD (9 of 16, 56%), pretransplant evaluation of pulmonary hypertension (2 of 16, 13%), or assessment of myocardial recovery (5 of 16, 31%). The median age at catheterization was 12 years (interquartile range: 8-16). Median baseline speed of device was 2333 ± 253 rotations per minute. The goal was to find the speed at which optimal hemodynamics were achieved, defined by low wedge pressure with an acceptable central venous pressure. Of the 16 catheterizations, there were 9 (56%) speed increases to achieve optimal hemodynamics and 5 (33%) speed decreases for hemodynamic optimization or for potential explant. The speed was not changed in 2 (13%) catheterizations as the patients were determined to be at an optimal hemodynamic state. Overall, VAD settings were optimized in 75% (14 of 16) of hemodynamic catheterizations. There were no adverse events related to catheterization. Thus, we conclude that catheterization-based hemodynamic assessment is safe and effective for optimizing VAD speed and provides guidance on medical management in children supported on CF-VAD.

Global Longitudinal Strain Analysis of the Single Right Ventricle: Leveling the Playing Field.

BACKGROUND: All available echocardiographic methods to assess single systemic right ventricular systolic function have limitations. Subjective grading is prone to bias and varies among readers. Quantitative methods that require significant manual input, such as fractional area change (FAC), are often not reproducible. The aim of this study was to determine whether global longitudinal strain (GLS) is more reproducible than FAC and subjective grading in patients with systemic right ventricle among individual readers and across different levels of experience. METHODS: Clinically indicated echocardiograms from 40 patients with functional systemic right ventricles were assessed by five readers with varying reading experience: one sonographer, one cardiology fellow, and three attending cardiologists at different career stages. All readers were blinded to patient data and other reader responses. Each reader reviewed the same images for subjective grade (on a scale ranging from 1 [normal] to 8 [severely depressed]), right ventricular end-diastolic and end-systolic area measurements, and longitudinal strain analysis. A repeat analysis was performed under identical conditions after ≥2 weeks on all 40 patients. Inter- and intrareader reproducibility was assessed using intraclass correlation coefficients (ICCs). Correlations between responses were assessed using Spearman's correlation coefficient. RESULTS: The subjective method had fair to good reproducibility (ICC = 0.7; interquartile range [IQR], 0.60-0.72), while the FAC method was poor (ICC = 0.46; IQR, 0.39-0.51) among readers. Reproducibility for GLS was excellent (ICC = 0.88; IQR, 0.88-0.89). Intrareader reproducibility was excellent by subjective grading (ICC = 0.85; IQR, 0.73-0.88), poor by FAC (ICC = 0.63; IQR, 0.35-0.66), and excellent by GLS (ICC = 0.93; IQR, 0.88-0.96). Attending-level readers were more consistent with their subjective grading, while all readers were excellent with GLS. CONCLUSIONS: GLS is more reproducible than conventional methods at assessing systemic right ventricular systolic function among readers with different levels of experience. For most readers it was more consistent than their own subjective grades of right ventricular function. Laboratories staffed by multiple readers are likely to be more consistent in grading systemic right ventricular systolic function using GLS.

Hyperglycemia and diabetes mellitus in children with pancreatitis.

OBJECTIVE: To assess the risk factors for developing hyperglycemia and diabetes mellitus (DM) in children with pancreatitis. STUDY DESIGN: Patients (from infants to age 21 years) hospitalized with acute pancreatitis (AP), acute recurrent pancreatitis (ARP), and chronic pancreatitis were studied retrospectively. Subjects with known DM or cystic fibrosis before presentation with pancreatitis were excluded. RESULTS: A total of 176 patients met the study criteria. Of these, 140 had AP, 29 had ARP, and 7 had chronic pancreatitis. Severe pancreatitis was associated with hyperglycemia; 41% of the patients with hyperglycemia required insulin, and 8 patients (4.5%) developed DM requiring insulin by the time of discharge. These 8 patients with postpancreatitis DM were more likely to be overweight. Five of the 8 patients had a seizure disorder, and 4 had another comorbidity, such as mental retardation or cerebral palsy. Seven of the 8 patients who developed DM had a single episode of AP, and one patient had ARP. CONCLUSIONS: Our findings indicate that hyperglycemia and DM can occur with pancreatitis. In some cases, postpancreatitis DM was associated with mental retardation, seizure disorder, and use of antiseizure medication. As opposed to adults who develop DM after chronic pancreatitis, children can develop DM due to a single episode of AP.

Dobutamine stress cardiac MRI is safe and feasible in pediatric patients with anomalous aortic origin of a coronary artery (AAOCA).

BACKGROUND: Risk stratification in anomalous aortic origin of a coronary artery (AAOCA) is challenged by the lack of a reliable method to detect myocardial ischemia. We prospectively studied the safety and feasibility of Dobutamine stress-cardiac magnetic resonance (DSCMR), a test with excellent performance in adults, in pediatric patients with AAOCA. METHODS: Consecutive DSCMR from 06/2014-12/2019 in patients≤20 years old with AAOCA were included. Hemodynamic response and major/minor events were recorded. Image quality and spatial/temporal resolution were evaluated. Rest and stress first-pass perfusion and wall motion abnormalities (WMA) were assessed. Inter-observer agreement was assessed using kappa coefficient. RESULTS: A total of 224 DSCMR were performed in 182 patients with AAOCA at a median age of 14 years (IQR 12, 16) and median weight of 58.0 kg (IQR 43.3, 73.0). Examinations were completed in 221/224 (98.9%), all studies were diagnostic. Heart rate and blood pressure increased significantly from baseline (p < 0.001). No patient had major events and 28 (12.5%) had minor events. Inducible hypoperfusion was noted in 31/221 (14%), associated with WMA in 13/31 (42%). Inter-observer agreement for inducible hypoperfusion was very good (Κ = 0.87). Asymptomatic patients with inducible hypoperfusion are considered high-risk and those with a negative test are of standard risk. CONCLUSIONS: DSCMR is feasible in pediatric patients with AAOCA to assess for inducible hypoperfusion and WMA. It can be performed safely with low incidence of major/minor events. Thus, DSCMR is potentially a valuable test for detection of myocardial ischemia and helpful in the management of this patient population.

Left ventricular strain and left atrial strain are impaired during hemodialysis in children.

We aimed to investigate intradialytic changes in ventricular and atrial function using speckle tracking echocardiography (STE) in pediatric hemodialysis (HD). Children with HD vintage > 3 months were enrolled, and echocardiography was performed prior to, during, and after HD. STE was analyzed using GE EchoPAC. Left ventricular (LV) global longitudinal strain (GLS), strain rate (Sr), and mechanical dispersion index (MDI) were calculated as the average from 3 apical views; diastolic strain (Ds) and Sr from 4-chamber tracing; left atrial strain (LAS) and Sr from the 4- and 2-chamber views. A total of 15 patients were enrolled at a median age of 12 years (IQR 8, 16) and median HD vintage of 13 months (IQR 9, 25). GLS worsened during HD (- 15.8 ± 2.2% vs - 19.9 ± 1.9%, p < 0.001). Post-HD GLS was associated with BP decrease (coefficient = 0.62, p = 0.01). LV MDI and systolic Sr did not change. LV Ds progressively worsened (- 8.4% (- 9.2, - 8.0) vs - 11.9% (- 13.4, - 10.3), p < 0.001). LAS changes at mid-HD returned to baseline post-HD. Ds, DSr, LAS, LASr were not associated with BV removal or BP decrease (p > 0.1). In conclusions, intradialytic LV strain and LAS changes consistent with subclinical systolic and diastolic dysfunction were observed during HD in children. Changes in Ds, DSr, LAS, and LASr were not associated with BP change or BV removal and may be related to the disease progression. Longitudinal study using these novel indices may unfold the effect of these subclinical changes on long-term cardiovascular health in children requiring chronic HD.